Osteofibrous Dysplasia-Like Adamantinoma of the Tibia Diagnosed at Age 6 Years Progressing into a Classic Adamantinoma over 39 Years, Reconstruction Performed by a Modified Capanna Technique: A Case Report

Background: It is generally accepted that there is a spectrum ranging from Osteofibrous Dysplasia (OFD) to Osteofibrous dysplasia-like Adamantinoma (OFD-AD) to “classic” Adamantinoma (AD). However, it is controversial whether OFD may progress into OFD-AD and AD or these pathologic changes reflect sampling issues. We herein report the fifth case of late development of AD from OFD-AD to underline the importance of long-term follow-up. Case: At 6 years bone biopsy of the tibia revealed the diagnosis of OFD-AD, confirmed by repeat biopsies until the age of 18 years. At age 45 years a progressive focal osteolysis was biopsied and showed histologically a classic AD. The affected tibial diaphysis was resected and pasteurized. Reconstruction was performed adapting the “Capanna technique” of inserting the ipsilateral mobilized fibula into the replanted autologous, pasteurized tibial segment as biologic stabilizer. At 1.5 years follow-up, the reconstruction has healed allowing full weight bearing.

角蛋白系列抗体在骨性纤维结构不良诊断中的意义

目的探讨免疫组化角蛋白系列抗体在骨性纤维结构不良(osteofibrous dysplasia,OFD)诊断及鉴别诊断中的意义。方法收集38例OFD及30例纤维结构不良(fibrous dysplasia,FD)、3例OFD样釉质瘤,通过光镜观察及免疫组化标记角蛋白系列抗体(CKpan、CK5/6、CKHMW、CK19、CK7、CK20、CK8、CK18)进行对比分析。结果 38例OFD中均可见散在角蛋白阳性的细胞,CKpan、CK5/6、CKHMW和CK19阳性率分别为100%、100%、100%、65.8%,且高分子量角蛋白表达较强,低分子量角蛋白CK7、CK20、CK8、CK18均阴性。30例FD中角蛋白抗体均阴性。3例OFD样釉质瘤中均可见角蛋白巢状阳性细胞。结论免疫组化出现散在角蛋白抗体阳性细胞可用于鉴别OFD与FD,角蛋白阳性细胞呈巢状时应诊断为OFD样釉质瘤。高分子量角蛋白可更好的显示OFD中的阳性细胞。